A case of unilateral and spontaneously resolving posterior uveitis with overlapping features of Vogt-Koyanagi-Harada disease and Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Abstract

Introduction: VKH disease is a chronic, bilateral, granulomatous panuveitis with potential involvement of neurological, auditory and integumentary systems. On the other hand, APMPPE is believed to be an immune-driven chorioretinal vascular disease characterized by multifocal, flat, grey-white placoid lesions at the level of the RPE. We describe a case with overlapping figures of both conditions.

Case description: A 19-year-old female presented with unilateral blurry vision and was found to have clinical and IVFA findings consistent with APMPPE. Her OCT study demonstrated typical VKH findings with large areas of serous neurosensory retinal detachment and intra-retinal cystoid spaces with enclosed membranous structures. She was closely followed but was not treated with high dose corticosteroid. Spontaneous and complete resolution of her symptoms and clinical, IVFA and OCT findings were achieved by day 25.

Discussion: This is the first reported case of spontaneously resolving, unilateral VKH disease in the absence of high dose corticosteroid treatment with overlapping features of APMPPE.

Conclusions: The imaging and clinical findings of both VKH disease and APMPPE raise the notion that VKH disease and APMPPE could be an overlapping spectrum of inflammatory processes, rather than distinct disease entities.

Keywords: APMPPE; Acute Posterior Multifocal Placoid Pigment Epitheliopathy; IVFA; OCT; Uveitis; VKH; Vogt–Koyanagi–Harada; White dot syndrome.

Fig. 1

Left eye fundus photograph at the time of disease onset. Multiple, creamy yellow, flat, placoid lesions at the level of retinal pigment epithelium and multiple areas of retinal elevation consistent with sub-retinal fluid were visualized in the posterior pole

Fig. 2

Wide-angle IVFA of the left eye at time of disease onset. a Arteriovenous phase showing diffuse areas of hypofluorescence more numerous than lesions seen on color fundus photograph. b Late phase showing late staining and leakage from areas of previous hypofluorescence and pooling over areas of serous retinal detachment

Fig. 3

OCT images during acute, resolving and resolved phases. a Day 0 of disease onset, areas of serous neurosensory detachment with subretinal fluid, ellipsoid layer and external limiting membrane disruption, attenuation and edema of photoreceptor segments were seen. b Day 4, large intraretinal cystoid spaces and membranous structures within the cystoid space were seen. c Day 12, significant resorption of subretinal fluid and cystoid spaces, as well as granular appearance of the membranous structure within the cystoid spaces were observed. There was also resolving edema and reduced disruption of the ellipsoid layer and photoreceptor segments. d Day 25, complete resorption of cystoid spaces and re-establishment of retinal layers